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Biomedical subjects

M A Pelli

Publications and source records attributed to M A Pelli.

63 records · Page 4Linked to original sources

Dopamine uptake by platelets in hepatic encephalopathy; evidence for a possible depletion of brain dopamine.

A decrease in H3-dopamine uptake was demonstrated in the blood platelets of 22 hepatic encephalopathy (HE) patients when compared to that of patients with liver cirrhosis, but without HE, and controls. There was a direct correlation between the stage of HE and the decrease in H3-dopamine uptake. As blood platelets have characteristics similar to neurons which contain amines, they have been proposed as a model for the study of amine metabolism in neurological, as well as liver diseases. A defective dopamine uptake by the HE platelets suggests that a similar biochemical derangement is, also, present in the nerve cells of the dopaminergic system. This could account for the clinical evidence of extrapiramidal dysfunction and the arousal effect of levodopa in HE. Platelets from 10 cirrhosis, but HE-free, patients had a dopamine uptake which was intermediate between the HE patients and controls. When octopamine was added at the same concentrations as in serum of HE patients, the blood platelets from five controls showed a decrease in dopamine uptake proportional to the concentration of octopamine added. Octopamine may impair dopamine uptake by platelets from HE patients.

Adult↗

Low-amplitude propagated contractile waves: a relevant propulsive mechanism of human colon.

BACKGROUND: Human colonic motility is still poorly understood, especially as far as concerns its propulsive function. Available data refer almost exclusively to the forceful propulsive activity, which is recognized as high-amplitude propagated contractions, the manometric equivalent of mass movements. By contrast, information on less vigorous propulsive contractions is still lacking. AIMS: To investigate the presence and behaviour of low-amplitude propagated contractile waves (less than 50 mmHg in amplitude) in the colon of healthy humans during a 24-hour study period. SUBJECTS AND METHODS: A series of 16 healthy volunteers of both sexes entered the study, and were investigated by a standard technique involving a colonoscopically-positioned manometric catheter. During the study, two standard 1,000 kcal mixed meal and a 450 kcal breakfast were served. The recordings were, therefore, scanned for the presence of low-amplitude propagated contractile waves (waves of less than 50 mmHg in amplitude, propagated over at least three consecutive recording ports), their daily distribution, and their relationship with physiological events. RESULTS: Low-amplitude propagated contractile waves were constantly present in all the tracings, with an average of about 61 events/subject/day and a mean amplitude of about 20 mmHg. More than 80% of these events appeared during the day, with a significant (p<0.05) increase after meals and after morning awakening. In 25% of subjects, these waves were accompanied by emission of flatus. CONCLUSIONS: In the human colon, low-amplitude propagated contractile waves are a constant physiological propulsive pattern, which is generally related to sleep-wake cycles and meal ingestion.

Adult↗

Epilepsy with bilateral occipital calcifications: Sturge-Weber variant or a different encephalopathy?

A series of cases of epilepsy with associated bilateral occipital calcifications (EBOC) without signs of phakomatosis and without any disorders known to produce cerebral calcifications have been reported. It is unclear whether EBOC is an incomplete variant of Sturge-Weber disease (SWD) or if it is a different, as yet undefined encephalopathy. We describe four new cases of EBOC that are different clinically by age of onset, type, course, severity of epilepsy, and associated cognitive deficits but that are linked by similar neuroradiologic findings. Similar to cases described in the literature, there is convincing evidence in favor of the hypothesis that these cases belong to an encephalopathy different from SWD and frequently associated with celiac disease.

Adolescent↗

[Changes in upper gastrointestinal motility during scleroderma].

Scleroderma (progressive systemic sclerosis) is a systemic collagen disease in which the upper gut is frequently involved. In particular, most patient show altered esophageal motility, which frequently result in severe esophagitis, often resistant to therapeutic measures. The small bowel is also frequently involved by the disease, especially in the late stage of scleroderma. Small bowel alterations are sometimes clinically silent, but can also be the origin of malabsorption syndrome, small intestine perforation, pneumatosis cystoides or chronic intestinal pseudo-obstruction. The occurrence of an altered gastrointestinal motility in scleroderma can be detected by means of manometric techniques; their use in the wide area of collagenopathies may help understanding the pathophysiology of the altered gastrointestinal function frequently existing in these diseases.

Esophagus↗

[Changes in the level of erythrocyte phosphorylated compounds in patients with hepatic encephalopathy].

Automated ion exchange chromatography was used to compare red cell phosphorylates in clinically healthy subjects and 6 patients with hepatic encephalopathy. Significant changes in the distribution of these compounds were noted, including a marked increase in total acid-soluble content (particularly 2.3 DPG, R5P, 3MPG and G16DP) and a sharp fall in ATP. Increased 2.3 DPG explained the rightward shift of the haemoglobin dissociation curve seen in cirrhosis of the liver, but does not seem to fit in with enhanced blood ammonia. Deep-seated changes in red cell energy metabolism may have the same pathogenesis as the CNS metabolic change observed in hepatic encephalopathy.

Adenine Nucleotides↗

Severe osteomalacia due to gluten-sensitive enteropathy.

This report discusses a severe case of osteomalacia due to gluten-sensitive enteropathy: it stresses the clinical features and describes an atypical form of gluten-sensitive enteropathy, in which gastroenterological symptoms were absent. Wasting and osteomalacia causing skeletal deformation with spontaneous fractures were observed in a 31-year-old woman who had marked hypophosphoremia, a tendency to low serum calcium levels and slight multi-deficiency anaemia. The patient was in a state of depression. The causes of osteomalacia and then a general malabsorption syndrome were investigated. Anti-gliadin antibodies were positive. Histological tests on duodenal mucous revealed a pattern indicative of gluten-sensitive enteropathy. A gluten-free diet was prescribed and at a check-up one month later the patient had improved markedly. Skeletal symptoms are predominant in 30% of atypical forms of gluten-sensitive enteropathy. The severity of this case was due to a late diagnosis.

Acute Disease↗

[Chronic therapy with ursodeoxycholic acid in a child with Alagille syndrome].

The effectiveness of oral bile acid therapy with ursodeoxycholic acid (10 mg/kg/day) was investigated in a 10 year old boy affected by Alagille's Syndrome, a chronic cholestatic disorder due to congenital hypoplasia of the intrahepatic biliary ducts. Cholestatic and hepatonecrotic indices were measured before and during ursodeoxycholic acid therapy and 1 month after stopping and 36 months after restarting the treatment. Ursodeoxycholic acid led to a marked improvement in the cholestatic and hepatonecrotic parameters which was maintained during all the treatment phase. Pruritus and steatorrhea disappeared during the treatment with ursodeoxycholic acid. Histological examination of the liver biopsy after the treatment revealed a disappearance of the biliary plugs but without increasing he intrahepatic bile ducts. The results suggest that ursodeoxycholic acid may improve the condition of the children affected by Alagille's Syndrome, specially when the liver transplantation is required, and indicate a need for long term studies in a larger number of patients.

Bile Ducts, Intrahepatic↗